Nuclear respiratory factors and the pathways of nuclear-mitochondrial interaction

Richard Scarpulla*

*Corresponding author for this work

Research output: Contribution to journalShort survey

57 Citations (Scopus)

Abstract

Mitochondrial oxidative pathways can be adversely affected by mutations in both nuclear and mitochondrial genomes. Recent evidence indicates that cardiac impairment is an important clinical feature of mitochondrial diseases resulting from such mutations. Understanding the regulatory interplay between nuclear and mitochondrial genetic systems may yield new insights into human genetic defects affecting cardiac function. Nuclear respiratory factors (NRF- 1 and NRF-2) are transcriptional activators that act on a significant subset of nuclear genes required for mitochondrial respiration. These factors most likely participate in nuclear-mitochondrial interactions by helping to coordinate the synthesis of respiratory chain subunits with components of the mitochondrial transcription, replication, and heme biosynthetic machinery. Thus, NRFs and related factors are likely contributors to the nuclear control of mitochondrial energy production that is essential for normal myocardial function.

Original languageEnglish (US)
Pages (from-to)39-45
Number of pages7
JournalTrends in Cardiovascular Medicine
Volume6
Issue number2
DOIs
StatePublished - Jan 1 1996

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Nuclear Respiratory Factors
Nuclear Respiratory Factor 1
Mitochondrial Diseases
Mutation
Mitochondrial Genome
Mitochondrial Genes
Medical Genetics
Electron Transport
Heme
Respiration

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

Cite this

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abstract = "Mitochondrial oxidative pathways can be adversely affected by mutations in both nuclear and mitochondrial genomes. Recent evidence indicates that cardiac impairment is an important clinical feature of mitochondrial diseases resulting from such mutations. Understanding the regulatory interplay between nuclear and mitochondrial genetic systems may yield new insights into human genetic defects affecting cardiac function. Nuclear respiratory factors (NRF- 1 and NRF-2) are transcriptional activators that act on a significant subset of nuclear genes required for mitochondrial respiration. These factors most likely participate in nuclear-mitochondrial interactions by helping to coordinate the synthesis of respiratory chain subunits with components of the mitochondrial transcription, replication, and heme biosynthetic machinery. Thus, NRFs and related factors are likely contributors to the nuclear control of mitochondrial energy production that is essential for normal myocardial function.",
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Nuclear respiratory factors and the pathways of nuclear-mitochondrial interaction. / Scarpulla, Richard.

In: Trends in Cardiovascular Medicine, Vol. 6, No. 2, 01.01.1996, p. 39-45.

Research output: Contribution to journalShort survey

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AB - Mitochondrial oxidative pathways can be adversely affected by mutations in both nuclear and mitochondrial genomes. Recent evidence indicates that cardiac impairment is an important clinical feature of mitochondrial diseases resulting from such mutations. Understanding the regulatory interplay between nuclear and mitochondrial genetic systems may yield new insights into human genetic defects affecting cardiac function. Nuclear respiratory factors (NRF- 1 and NRF-2) are transcriptional activators that act on a significant subset of nuclear genes required for mitochondrial respiration. These factors most likely participate in nuclear-mitochondrial interactions by helping to coordinate the synthesis of respiratory chain subunits with components of the mitochondrial transcription, replication, and heme biosynthetic machinery. Thus, NRFs and related factors are likely contributors to the nuclear control of mitochondrial energy production that is essential for normal myocardial function.

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